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Indian Pediatr 2019;56: 701-702

Neonatal Lupus with Extensive Cutaneous Involvement


Vibhu Mendiratta and Anuja Yadav*

Department of Dermatology and STD, Lady Hardinge Medical College, New Delhi, India.

Email: * [email protected]

 

 


A 2-month-old girl presented with multiple hypo-pigmented and depressed lesions over face, scalp and erythematous lesions over forehead, cheeks and around eyes, since birth. The mother was asymptomatic at the time of presentation with no history of drug intake during pregnancy, preeclampsia, photosensitivity or recurrent oral ulcers. On cutaneous examination, multiple depressed, atrophic and hypopigmented plaques were present on face involving forehead, cheeks, periorbital area and scalp (Fig. 1), anterior abdomen and back. Investigations revealed anemia (hemoglobin 8.9 g/dL), thrombocytopenia (platelet count, 90×10
9/L) and raised liver enzymes. Antinuclear antibody (ANA) test was positive (2+); speckled pattern and anti-Ro La was present in high titers in both mother and infant. VDRL of mother and baby was non-reactive. Ultrasound abdomen, electrocardiogram and echocardiography were normal. We diagnosed neonatal lupus erythematosus, and advised tacrolimus 0.03% ointment once daily with strict photo-protection. The patient showed marked improvement in the lesions in the next three months.

Fig. 1 Multiple depressed, atrophic and hypopigmented plaques on forehead, cheeks, periorbital area and scalp.

Neonatal lupus erythematosus (NLE) is a rare, often misdiagnosed immune-mediated disease. It is usually associated with maternal antibodies to proteins Ro/ La (SSA/SSB) which are transferred by the placenta. Differential diagnoses of NLE include tinea corporis (centrifugal skin lesions with more inflammation at periphery), seborrheic dermatitis (scaly and yellowish round or annular pattern of lesions), congenital syphilis (positive VDRL test), annular erythema of infancy, and aplasia cutis (absence of skin since birth). Congenital heart block and cardiomyopathy are cardiac manifestations of NLE. Management of cutaneous lesions include photo-protection and low-potency topical corticosteroids. Systemic corticosteroids, intravenous immunoglobulins, and immunosuppressive agents have also been tried. Majority of patients with NLE of the skin, liver or blood have transient disease that spontaneously resolves after 4-6 months, but can have substantial morbidity and mortality if there is congenital heart block.

 

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